Clinical manifestation of esophageal carcinosarcoma: a Taiwan experience.

Carcinosarcoma of the esophagus is a rare neoplasm with both carcinomatous and sarcomatous components. This study aimed to investigate its clinicopathologic features and endoscopic characteristics. The data of patients diagnosed to have esophageal carcinosarcoma pathologically in the past 30 years (January 1976-December 2007) were reviewed. Of 3318 cases of esophageal malignancy, 12 were diagnosed as esophageal carcinosarcoma, with an incidence of 0.36%. All of the cases were male with a mean age of 62.3 years. Of the 12 tumors, 8 were polypoid type, and 4 were ulcerative type. In the endoscopic ultrasonography examination, the tumors show heterogeneous hypoechoic lesions with irregular outer margins and internal multicystic components. Four patients (33.3%) had previous head and neck squamous cell carcinoma that occurred metachronously. This is the first report about the characteristics of esophageal carcinosarcoma under endoscopic ultrasonography examination. The relationship between esophageal carcinosarcomas and head and neck cancer needs further investigation.

Natural history of hepatitis C virus infection in multitransfused hemophiliacs: effect of coinfection with human immunodeficiency virus. The Multicenter Hemophilia Cohort Study.

The objective of this prospective cohort study was to describe the natural history of hepatitis C virus (HCV) infection and the effect of human immunodeficiency virus (HIV) on the clinical manifestations of HCV liver disease. Two hundred twenty-three hemophiliacs were followed in a comprehensive care setting with periodic clinical and laboratory evaluations. Dates of HIV seroconversion were determined retrospectively from frozen sera. HCV assays were performed by a "second generation" four-antigen recombinant immunoblot assay (RIBA 2). Liver failure was found after a latency period of 10 to 20 years in 9% of multitransfused HCV-positive/HIV-positive adult hemophiliacs without an AIDS-defining opportunistic infection or malignancy. Lymphocytopenia, decreased CD4 counts, and, possibly, thrombocytopenia were associated with liver failure which appeared to be accelerated by HIV disease and its treatment. This form of severe liver disease is being seen with increasing frequency among multi-transfused persons with hemophilia who are coinfected with HCV and HIV.

Mutational analysis of the p27(kip1) gene in hepatocellular carcinoma.

p27(Kip1) is an inhibitor of cyclin-dependent kinase. It has been reported that reduced p27(Kip1) expression is present in human hepatocellular carcinoma. To determine the role of p27(Kip1) in hepatocarcinogenesis, 46 cases with hepatocellular carcinomas were studied. p27(Kip1) mutation was first screened by single strand conformation polymorphism, and direct DNA sequencing was then performed on those cases with mobility shifts. Two polymorphism sites were found. One is a previously described polymorphism at codon 109 (GTC-->GGC) which was found in two cases. The second polymorphism was identified at codon 55 (GCG-->GCA) in six of the 46 cases. However, the polymorphism at codon 55 was also present in seven of 93 healthy controls (7.5%), indicating that it is not associated with a predisposition for development of hepatocellular carcinoma (Fisher's exact test, 0.05). These results show that p27(Kip1) mutation is not a frequent event in human hepatocellular carcinoma, and suggest that it may be inactivated predominantly by transcriptional and/or posttranscriptional regulation rather than genomic aberrations.

Term early-onset neonatal seizures: obstetric characteristics, etiologic classifications, and perinatal care.

OBJECTIVE: To describe obstetric characteristics and etiologic classifications and assess perinatal care in term neonates with early-onset seizures. METHODS: We performed a retrospective review of neonatal and obstetric records of neonates delivered at term with a diagnosis of early-onset seizures between January 1981 and December 1992 at Long Beach Memorial Medical Center. Data regarding obstetric characteristics and etiologic classifications of the seizures were abstracted from the medical records. Lack of antepartum testing in high-risk patients, delayed intervention with nonreassuring antepartum or intrapartum fetal heart rate patterns, birth trauma, and failure to use prophylactic antibiotics or treat infection were the criteria used for identifying seizures that were potentially preventable. RESULTS: Forty term neonates had early-onset seizures out of 60,712 live births (0.07%). These seizures were attributed to hypoxic events in 15 neonates (37.5%), cerebral malformations in seven (17.5%), cerebral infarcts in seven (17.5%), intracranial hemorrhage in five (12.5%), infection in three, and an unknown etiology in three. Twenty-three neonates had 5-minute Apgar scores of 7 or greater (cerebral malformations excluded). Seven of these neonates (30%) had cerebral infarcts. A review of all records identified nine cases (22.5%) of the early-onset seizures as potentially preventable. CONCLUSION: The majority of the term early-onset neonatal seizures identified did not appear to be preventable. Many of the neonates with 5-minute Apgar scores of 7 or greater had cerebral infarcts.

Angiomyolipoma of the liver presenting in pregnancy.

Primary angiomyolipoma of the liver is a rare entity with fewer than 40 cases described in the English literature. Primary hepatic tumors diagnosed during pregnancy are also rare with adenomas, hemangiomas, and hepatocellular carcinoma having been described. This article reports a patient who was diagnosed with a large liver mass at 16 weeks gestation. The patient successfully completed her pregnancy and ultimately underwent resection of a giant hepatic angiomyolipoma. The literature on angiomyolipoma as well as liver tumors during pregnancy is reviewed.

Extracorporal liver support with molecular adsorbents recirculating system in patients with hepatitis B-associated fulminant hepatic failure.

Hepatitis B virus (HBV) infection is the most prevalent cause of fulminant hepatic failure (FHF) in the Far East. HBV-associated FHF is characterised by rapidly progressive end organ dysfunction/failure and a very poor prognosis. To investigate how molecular adsorbent recirculating system (MARS) treatment impacts multiple organ system function in HBV-associated FHF. Ten consecutive patients were treated with MARS in a period of 12 months. Clinical, biochemical and haemodynamic parameters were assessed before and after MARS. Various disease severity scoring systems including model for end-stage liver disease, APACHE II, APACHE III, sequential organ failure assessment and organ system failure scores were also assessed. There were significant improvements in hepatic encephalopathy grading (p < 0.001), mean arterial pressure (p < 0.001), plasma renin activity (p = 0.027), bilirubin (p < 0.001), ammonia (p = 0.001) and creatinine levels (p < 0.001). There were also significant improvements in all the scoring systems evaluated. Meanwhile, platelet count was significantly decreased (p < 0.001). One patient was successfully bridged to liver transplantation. Three patients were alive at 3 months of follow-up. MARS can improve multiple organ functions in HBV-associated FHF. On the basis of these findings, randomised controlled studies are indicated and justified.

Spontaneous liver hematoma in pregnancy not clearly associated with preeclampsia: a case presentation and literature review.

Spontaneous liver hemorrhage with formation of subcapsular hematomas and rupture of Glissan's capsule is a rare but often lethal complication of pregnancy. This entity has usually been associated with severe preeclampsia or the HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome. A case of spontaneous subcapsular hematoma of the liver occurring in the third trimester is presented in which the patient probably had neither preeclampsia nor the HELLP syndrome. The literature on liver hematomas in pregnancy published since 1982 when the term HELLP syndrome was coined is reviewed with a focus on the association of liver hematomas with preeclampsia and the HELLP syndrome. Therapy and maternal and neonatal outcomes for this entity are reassessed.

Evidence that a capped oligoribonucleotide is the primer for duck hepatitis B virus plus-strand DNA synthesis.

The plus strand of virion DNA of duck hepatitis B virus possessed, at its 5' terminus, a capped oligoribonucleotide 18 to 19 bases in length. This oligoribonucleotide had a unique 5' end, the heterogeneity in length reflecting two distinct junctions with plus-strand DNA that were 1 base apart. The sequence of the RNA differed from that predicted by the sequence of duck hepatitis B virus upstream of the 5' ends of plus-strand DNA but was identical to a downstream sequence corresponding to the 5' terminus of a major poly(A)+ viral RNA mapped by Büscher and co-workers (Cell 40:717-724, 1985). This RNA transcript is thought to serve as the template (i.e., the pregenome) for minus-strand synthesis via reverse transcription. The results suggest that the pregenome also donates a capped oligoribonucleotide that acts as the primer of plus-strand DNA synthesis, using the minus-strand DNA as template.

Initiation and termination of duck hepatitis B virus DNA synthesis during virus maturation.

We characterized a number of important features of the structure of the cohesive overlap region of the DNA genome of duck hepatitis B virus. The 5'-terminal nucleotide of minus-strand DNA was localized to nucleotide 2537, a G residue within the 12-base repeat sequence DR1. This G residue was shown to be the site of a covalent linkage to a protein, consistent with speculation that this protein is the primer of minus-strand synthesis, which occurs by reverse transcription. The 3' terminus of the minus strand was heterogeneous, being mapped to nucleotides 2530 and 2531, indicating that the minus strand is terminally redundant by seven or eight bases and ends at the putative 5' end of the transcribed RNA template (pregenome) for reverse transcription. We previously demonstrated that the presumptive RNA primer of plus-strand synthesis remains attached to plus-strand DNA during virus maturation; moreover, the sequence of this primer suggested an origin from the 5' end of the pregenome (J.-M. Lien, C. E. Aldrich, and W. S. Mason, J. Virol. 57:229-236, 1986). We show here that over 75% of plus-strand primers are capped, further supporting the idea that these primers are uniquely derived from the 5' end of the pregenome. Finally, we found that seemingly mature duck hepatitis B virus genomes are incomplete by at least 12 bases, in that the 12-base repeat sequence DR2 is not copied into plus-strand DNA during virus maturation. Since DR2 in virion DNA is duplexed with the RNA primer of plus-strand synthesis, it is possible that the failure to make complete plus strands is due to an inability of the viral DNA polymerase to carry out a displacement of the bound RNA primer.

Combined endoscopic and surgical treatment for the polyposis of Peutz-Jeghers syndrome.

Repeated laparotomy with extensive small bowel resectioning and eventual short-bowel syndrome is a major problem in Peutz-Jeghers syndrome (PJS) patients. This problem is caused by gastrointestinal polyposis with intussusception. A combined surgical and endoscopic approach can assess the extent of the polyposis, and small polyps can be removed by snare polypectomy. This can avert multiple enterotomies and decrease bowel resection segments. We applied an intraoperative colonscope via the enterotomy route in an 20-year-old PJS woman, and successfully removed the other 10 polyps distributed in the whole small bowel. As part of an aggressive approach to the management of polyposis in PJS, complete polypectomy can provide a longer symptom-free interval and remove potentially premaligment polyps.

Pulmonary injury associated with appendicitis in pregnancy: who is at risk?

OBJECTIVE: Our purpose was to determine risk factors for pulmonary injury in women with antepartum appendicitis. STUDY DESIGN: This case-control study included 49 patients with appendicitis during pregnancy. Patients who had pulmonary injury composed the study group (n = 9); the control subjects had no injury (n = 40). Records were abstracted for gestational age at surgery, type of anesthesia, presenting symptoms, findings on physical examination, vital signs, laboratory test results, use of tocolytics or antibiotics, and fluid management. Pulmonary injury was characterized by dyspnea, tachypnea, PaO2 < or = 70 mm Hg, and an abnormality on chest radiography. RESULTS: Pulmonary injury developed in nine study patients (18%) (adult respiratory distress syndrome, n = 2; pulmonary edema or infiltrates, n = 7) as a complication of appendicitis during pregnancy. All study group patients were at > 20 weeks' gestation compared with 27 of 40 (67%) control subjects (p = 0.05). Univariate analysis showed that fluid overload > or = 4 L, maximum respiratory rate > 24 breaths/min, maximum heart rate > 110 beats/min, maximum temperature > or = 100.4 degrees F, general anesthesia, and tocolytic use were significant (p < 0.005). By multivariate analysis with the use of stepwise logistic regression a model of fluid overload > or = 4 L, respiratory rate > 24 breaths/min, maximum temperature > or = 100.4 degrees F, and tocolytic usage would predict 99% of injury cases. CONCLUSIONS: Iatrogenic factors such as injudicious fluid management and tocolytic use can greatly increase the risk for pulmonary injury with antepartum appendicitis.

The value of amniotic fluid interleukin-6 determination in patients with preterm labor and intact membranes in the detection of microbial invasion of the amniotic cavity.

OBJECTIVES: Our purpose was to (1) determine the value of amniotic fluid interleukin-6 determination in the detection of microbial invasion of the amniotic cavity and (2) compare interleukin-6 to other rapid tests in the evaluation of preterm labor. STUDY DESIGN: Amniotic fluid interleukin-6 was determined quantitatively by enzyme-linked immunosorbent assay in 91 amniotic fluid specimens obtained by amniocentesis in 89 patients with preterm labor. Amniotic fluid cultures for aerobes, anaerobes, and mycoplasma species were performed. Receiver-operator characteristic curve analysis, logistic regression analysis, and Cox's proportional-hazards model were used to explore the relationship between several explanatory and outcome variables. Diagnostic index values of interleukin-6, glucose level, Gram stain, leukocyte esterase, and limulus amebocyte lysate assay for prediction of a positive amniotic fluid culture, preterm delivery, clinical infection, and neonatal sepsis were calculated. RESULTS: The prevalence of positive amniotic fluid cultures was 13% (12/89). The median interleukin-6 concentration in patients with positive cultures was 241.8 ng/ml, in contrast to 0.291 ng/ml in patients with negative cultures (p. < 0.005). Sensitivity and specificity of an interleukin-6 level > or = 6.17 ng/ml was 75% and 79%, in contrast to that of glucose, < or = 12 mg/dl (83% and 86%) for a positive amniotic fluid culture and sensitivity (p = 0.26, z test). All patients with an interleukin-6 level > 6.17 ng/ml were delivered preterm, in contrast to 85.2% of patients with a glucose level < or = 12 mg/dl. When all rapid tests and clinical parameters were considered simultaneously in the logistic model, only interleukin-6 maintained a significant relationship to preterm birth (odds ratio 35, p = 0.003). Cox's proportional analysis demonstrated a strong relationship between interleukin-6 and the amniocentesis-to-delivery interval after clinical variables were controlled for (hazard ratio 3.01, p < 0.00001). CONCLUSION: Amniotic fluid interleukin-6 determination may be a useful adjunct to our armamentarium of rapid tests to exclude infection and predict delivery in patients with preterm labor and intact membranes.

Antepartum cervical ripening: applying prostaglandin E2 gel in conjunction with scheduled nonstress tests in postdate pregnancies.

OBJECTIVE: Our purpose was to evaluate whether inserting prostaglandin E2 gel at the time of scheduled nonstress tests in patients with postdate pregnancies can decrease rates of intervention. STUDY DESIGN: A multicenter pilot study enrolled women with postdate pregnancies with Bishop score < or = 6 who were undergoing antepartum fetal heart rate testing. Patients were randomized in a double-blind fashion to receive either a prostaglandin E2 intracervical gel (Prepidil) or a placebo gel after each of their scheduled nonstress tests. RESULTS: There were no significant differences in the number of antepartum tests, labor inductions, or cesarean sections, the maximum oxytocin dosage, or the interval from admission to delivery in the prostaglandin E2 gel and placebo gel groups (n = 90). In the subset of patients with a Bishop score between 3 and 6 (63 patients), there were fewer inductions in the prostaglandin E2 group (30% vs 55%, P < .05). CONCLUSION: Application of prostaglandin E2 gel at the time of scheduled antepartum testing in patients with postdate pregnancies with unfavorable cervices decreased the induction rate only among patients with intermediate Bishop scores.

Non-O1 Vibrio cholerae bacteremia in patients with cirrhosis: 5-yr experience from a single medical center.

OBJECTIVES: To assess the clinical features and susceptibility of cirrhotic patients to non-O1 Vibrio cholerae bacteremia and to provide our therapeutic experiences in this rare and high lethal infection. METHODS: Twenty-eight blood culture isolates of non-O1 V. cholerae were identified by our clinical microbiology laboratory between July 1989 and June 1994. Patients with underlying cirrhosis and the aforementioned bacteremia were retrospectively reviewed. RESULTS: Twenty-one cirrhotic patients (16 male, five female; mean age, 50.9 yr; range 28-67 yr) were identified and classified as Child B (6 cases) and Child C (15 cases). Bacteremic episodes occurred most often from March to September. Seafood ingestion (seven cases) and seawater exposure (two cases) were risk factors, but nosocomial infections were also noted in six cases. Presenting symptoms and signs included ascites (95.2%), fever (81%), abdominal pain (52.4%), diarrhea (33.3%), and cellulitis with bullae formation (19%). Concurrent spontaneous bacterial peritonitis was determined in 10 cases, seven with positive ascites cultures. Antibiotic therapy (either cephalothin with gentamicin or ceftriaxone alone) cured most of the bacteremic episodes. The overall case-fatality rate was 23.8%, but 75% of the deaths were observed in patients with skin manifestation. CONCLUSIONS: Patients with decompensated cirrhosis are susceptible to non-O1 V. cholerae bacteremia and should not ingest raw seafood or expose skin wounds to salt water. A high index of suspicion and early administration of antibiotics may lower the mortality rate.

Solitary extramedullary plasmacytoma in the retroperitoneum.

Solitary extramedullary plasmacytoma is an uncommon neoplasm and occurs most frequently in the upper respiratory tract. Herein, we reported a solitary extramedullary plasmacytoma in the retroperitoneum. A 28-year-old man presented with obstructive jaundice and a retroperitoneal tumor. Ultrasound-guided biopsy confirmed that the lesion was a plasma cell neoplasm. A detailed investigation showed that no other sites were involved. The tumor got a moderate reduction following local irradiation, and a complete remission was achieved after 12 courses of adjuvant chemotherapy. Therefore, the possibility of a solitary extramedullary plasmacytoma should be considered in the differential diagnosis of obstructive jaundice without a history of multiple myeloma.

Angiomyolipoma of the liver: case report.

Hepatic angiomyolipoma is a rare benign mesenchymal tumor of the liver. Most multiple hepatic angiomyolipomas have appeared in patients with renal angiomyolipoma and tuberous sclerosis. A 38-year-old female patient without chronic hepatitis B or C was hospitalized because of epigastric fullness for 2 months. Radiologic studies showed a large solid tumor with a small daughter nodule in the right hepatic lobe. Upon intravenous bolus injection of contrast medium, both tumors showed weak heterogeneous enhancement in the delayed phase. Although hepatocellular carcinoma was suspected by the findings of computed tomography, percutaneous transhepatic ultrasound-guided biopsy was performed for the large tumor. The histopathology showed many mature fat cells intermingled with thick-walled blood vessels, and epithelioid cells with eosinophilic cytoplasm; the epithelioid cells stained positively for HMB-45 and smooth muscle actin. Angiomyolipoma of the liver was confirmed. The main tumor enlarged considerably during a follow-up period of 3 years. Surgical resection was performed due to persistent symptoms. She had an uneventful postoperative recovery and was well when followed up 10 months after surgery. We should be aware that a hepatic angiomyolipoma can change in size during its natural course, and this finding does not necessarily indicate malignancy.

Hemosuccus pancreaticus from a traumatic gastroduodenal pseudoaneurysm: an unusual cause of upper gastrointestinal bleeding.

Hemosuccus pancreaticus, blood entering the gastrointestinal tract through the pancreatic duct, is a rare and elusive form of gastrointestinal bleeding with diagnostic difficulties. We report a 37-year-old man who had recurrent gastrointestinal bleeding from erosion of a pseudoaneurysm of the gastroduodenal artery into the pancreatic duct. The lack of history of pancreatitis, associated symptoms, equivocal endoscopic findings, and the rarity of this entity resulted in a delay in diagnosis. Pancreatic duct stenosis detected during surgery suggested the lesion might have been caused by blunt abdominal trauma. A nearly total pancreatectomy and splenectomy were performed. The patient remained symptom-free 8 months after the operation. This obscure cause of gastrointestinal bleeding should be considered when common causes of bleeding have been ruled out, even in the absence of pancreatitis.

Acute jejunogastric intussusception: report of five cases.

The purpose of this study was to investigate the clinical manifestations and diagnosis of patients with acute jejunogastric intussusception. From May 1986 to June 1999, a total of 5 men (54-76 years old) were collected. Their initial presentations included epigastralgia (4), coffee-ground vomitus (3), frank hematemesis (1), and tarry stool (1). All patients had gastric surgeries 10-30 years previously. Radiograph of the abdomen showed a soft tissue density at the left upper quadrant in one patient. Panendoscopies were done in 4 patients. An obstructed efferent loop with a distended hyperemic small bowel protruding into the remnant of stomach was found in 3 cases, gangrenous change of the bowel wall in one of them. Stump cancer was diagnosed initially in the other patient. Barium study (3/5) showed efferent loop obstruction with "coil spring sing" and a central defect in the stomach. All 5 patients underwent segmental resection and end-to-end anastomosis between the 2nd to 6th hospital day. Operative findings were type II jejunogastric intussusception with retrograde invagination of a segment of efferent loop (30-100 cm in length) into the stomach. In conclusion, acute jejunogastric intussusception is an emergent condition. Early and accurate diagnosis is important. A high susception must be kept in mind in patients having a history of gastrojejunostomy with severe abdominal pain or upper gastrointestinal bleeding.

Postprandial versus preprandial blood glucose monitoring in women with gestational diabetes mellitus requiring insulin therapy.

BACKGROUND: The fetuses of women with gestational diabetes mellitus are at risk for macrosomia and its attendant complications. The best method of achieving euglycemia in these women and reducing morbidity in their infants is not known. We compared the efficacy of postprandial and preprandial monitoring in achieving glycemic control in women with gestational diabetes. METHODS: We studied 66 women with gestational diabetes mellitus who required insulin therapy at 30 weeks of gestation or earlier. The women were randomly assigned to have their diabetes managed according to the results of preprandial monitoring or postprandial monitoring (one hour after meals) of blood glucose concentrations. Both groups were also monitored with fasting blood glucose measurements. The goal of insulin therapy was a preprandial value of 60 to 105 mg per deciliter (3.3 to 5.9 mmol per liter) or a postprandial value of less than 140 mg per deciliter (7.8 mmol per liter). Obstetrical data and information on neonatal outcomes were collected. RESULTS: The prepregnancy weight, weight gain during pregnancy, gestational age at the diagnosis of diabetes and at delivery, degree of compliance with therapy, and degree of achievement of target blood glucose concentrations were similar in the two groups. The mean (+/- SD) change in the glycosylated hemoglobin value was greater in the group in which postprandial measurements were used (-3.0 +/- 2.2 percent vs. 0.6 +/- 1.6 percent, P < 0.001) and the infants' birth weight was lower (3469 +/- 668 vs. 3848 +/- 434 g, P = 0.01). Similarly, the infants born to the women in the postprandial-monitoring group had a lower rate of neonatal hypoglycemia (3 percent vs. 21 percent, P = 0.05), were less often large for gestational age (12 percent vs. 42 percent, P = 0.01) and were less often delivered by cesarean section because of cephalopelvic disproportion (12 percent vs. 36 percent, P = 0.04) than those in the preprandial-monitoring group. CONCLUSIONS: Adjustment of insulin therapy in women with gestational diabetes according to the results of postprandial, rather than preprandial, blood glucose values improves glycemic control and decreases the risk of neonatal hypoglycemia, macrosomia, and cesarean delivery.